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CLINRE-542; No. of Pages 5

Clinics and Research in Hepatology and Gastroenterology (2014) xxx, xxx.e1—xxx.e5

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LETTER TO THE EDITOR

Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis Dear editor, The deposition of amyloid within the gastrointestinal tract is a common characteristic of systemic amyloidosis [1]; however, primary gastric amyloidosis is extremely rare [2]. The prognosis for patients diagnosed with primary gastric localized amyloidosis (PGLA) remains poor, despite currently available non-specific therapeutic approaches, including administration of dimethyl sulfoxide (DMSO), nutritional support, and surgery. Unfortunately, none of these approaches yields significant benefits in clinical practice. A 33-year-old female patient presented with complaints of epigastric pain that had commenced 3 weeks prior. Initially, she developed epigastric discomfort and dyspepsia with heartburn and acid reflux, which was associated with occasional epigastric pain. Within a week of the onset of discomfort, the patient’s symptoms worsened. A physical examination and laboratory tests revealed no abnormalities. Endoscopic examination revealed a lesion with irregular borders and mucosa in the lesser curvature of the gastric body, measuring approximately 1.2 × 1.2 cm (Fig. 1A1—2 ). The lesion was associated with superficial, diffuse, small patches of hemorrhagic patches in the mucosa of the gastric body and fundus, and an additional lesion with regular borders and a smooth mucosal surface in the gastric fundus measuring 1.0 × 2.0 cm (Fig. 1B1—2 ). Hematoxylin and eosin staining revealed a structureless deposition of amyloid that extended from the submucosal layer to the muscularis propria, and large amounts of amyloid were also found around the small blood vessels of both lesions. The amyloid deposition was negative for van Gieson staining and positive for Congo red staining (Fig. 1). Multiple biopsies from the esophagus, duodenum, colon, and bone marrow demonstrated no amyloid deposition. Subsequently, the patient underwent an endoscopy ultrasound evaluation with a 7.5-MHz echoendoscope (UMP230; Olympus Optical Co. Ltd., Tokyo, Japan). A normal gastric wall was observed in five ultrasonographic layers. The lesion in the mucosa of the lesser curvature

of the gastric body had a hypoechoic stratum mucosum and lamina muscularis mucosae with increased thickness extruding into the gastric cavity. The local muscularis propria was intact. The second lesion in the gastric fundus was non-echogenic and was traceable through the submucosa of stratum mucosum (Fig. 2). Computed tomography imaging revealed a parenchymal goiter with a diffuse spindle shape on the posterior wall of the gastric body that measured 4.0 × 1.1 cm and was accompanied by a low-density cystic area (25 Hu; Fig. 2). A diagnosis of PGLA was made. An endoscopic submucosal dissection procedure was performed using a hook knife (KD-260LR; Olympus Optical Co. Ltd., Japan) and an IT knife (KD-610L; Olympus Optical Co. Ltd., Japan) with a high-frequency generator (ICC200; Erbe, Tubingen, Germany) through a standard single-accessory-channel endoscope (GIF-H260; Olympus Optical Co. Ltd., Japan), as described previously [3]. The margin of the lesion was defined by an argon-air knife. Marking dots were placed on the normal mucosa approximately 5 mm away from the lesion, and direct views of the submucosal layer were possible. Injections of saline and epinephrine were inserted into the submucosal layer around the lesion margin to elevate the mucosa [4]. A mucosal incision was then made outside the marking dots with a hook knife. Direct dissection of the submucosal layer beneath the lesion was performed under direct vision to obtain tissue samples using IT knife. During the endoscopic submucosal dissection (ESD) operation under general anesthesia, the exposed blood vessels of the lesion beds were coagulated thoroughly with diathermy forceps (Fig. 3). The lesions were harvested using aspiration and evaluated histologically. The aspirated lesions were similar to the pre-treatment biopsies and the diagnosis of PGLA was confirmed. After ESD, the patient was designated nil by mouth and received intravenous fluids and omeprazole (40 mg bid). Two weeks postoperatively, a repeat endoscopy revealed ulceration of the gastric mucosa (Fig. 4). DMSO was administered orally for 6 months after the ESD procedure (33.3% solution in water or fruit juice, batch number Q/HG314499; Tianjin Bodi Chemical Industry Co. Ltd., China) in accordance with previous reports [5]. The initial dose was 3.0 g/d, which was gradually increased up to the maximum tolerable dose of 20.0 g/d. The total dose was 3.0—20.0 g/d, and administered in three equal doses after meals. Follow-up

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Please cite this article in press as: Jin S-Z, et al. Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis. Clin Res Hepatol Gastroenterol (2014), http://dx.doi.org/10.1016/j.clinre.2014.01.002

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Figure 1 Endoscopic findings pre- and post-treatment and histological examination of biopsied gastric body and gastric fundus. A 1.2 × 1.2 cm irregular protuberance with an eroded irregular surface and borders was observed in the lesser curvature of the gastric body. Pathological samples were taken from here (A1 ). A 1.0 × 1.0 cm submucosal mass was found in the gastric fundus with a smooth surface in an area of good gastric mobility. The endoscope with a monocyclic COOK ligator was inserted into the stomach and targeted to the lesion. The surface mucosa of the tumor was dissected by the ligator, but the tumor was too large to be extracted and the endoscope was removed (B1 ). Scars with smooth surfaces and normal surrounding mucosa were observed at the gastric fundus (A2 ), lesser curvature of the gastric antrum and angle of the stomach (B2 ) postoperatively. Active bleeding and fistulae were not observed. The surface of the surrounding mucosa was smooth and its appearance was normal. An irregular protuberance with an eroded irregular surface and borders was observed in the gastric body (C1 ). The section showed that there was a glass-like deposition in the deep lamina propria of the gastric mucosa and submucosa, and some surrounding the small blood vessels (C2 ). The deposition stained positive for Congo red (C3 ) and van Gieson (C4 ) staining, and the medullary thyroid carcinoma tissue was used as a control. Compared to the control group, the deposition was considered to be a starch-based substance rather than collagen fibers.

Please cite this article in press as: Jin S-Z, et al. Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis. Clin Res Hepatol Gastroenterol (2014), http://dx.doi.org/10.1016/j.clinre.2014.01.002

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Figure 2 Ultrasonic endoscope results and contrast-enhanced CT images. A 1.0 × 1.0 cm anechoic area originating from the submucosa (A) was found in the gastric fundus lesion. A further 1.0 × 1.0 cm irregular protuberance (B) was observed in the lesser curvature of the gastric body. The mucosal layer and muscularis mucosae of the lesion were thickened, hypoechogenic and protruded into the stomach cavity. When the stomach was filled with water, a limited hybrid density shadow was observed in the gastric fundus, measuring 2.0 × 0.5 cm. The margin of the shadow was not clear and showed a low-density area, and the boundary with the spleen was not clear. The sidewall of the gastric curvature was thickened but there was no significant abnormal change during the triphase enhancement. (C) The gastric fundus was found to be cystic and showed no enhancement. The CT value was approximately 25 Hu, and the margin of the lesion was clear and closely related to the spleen. (D) The sidewall of the gastric curvature was locally thickened and there was no significant change during the triphase enhancement.

endoscopy at 6 months postoperatively showed a normal gastric mucosa and no signs of PGLA recurrence (Fig. 1). The patient was asymptomatic during the subsequent 18-month follow-up period. We describe a novel ESD procedure approach used in combination with oral DMSO to treat a patient with PGLA, with a successful curative outcome. Amyloidosis is a disorder of the deposition of amyloid in various tissues and organs, with

distinct features depending on the affected regions. PGLA can cause a variety of gastrointestinal symptoms, which contributes to the involvement of the gastric dysfunction and the damage to the structure of the gastric wall [6]. Currently, no efficacious specific therapeutic approach for PGLA is available, and only supportive measures are employed. DMSO may provide relief of digestive symptoms in patients with PGLA, and such treatment has been correlated with

Please cite this article in press as: Jin S-Z, et al. Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis. Clin Res Hepatol Gastroenterol (2014), http://dx.doi.org/10.1016/j.clinre.2014.01.002

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Figure 3 Endoscopic submucosal dissection. The lesion of the gastric curvature (approximately 1/4 of the total lesion area) was selected (A, B, C, F). An argon knife was used to mark the edge of the lesion, and the needle was inserted into the submucosa around the lesions. Saline with epinephrine was injected to uplift the lesions. A hook knife and IT knife were used to incise the mucosa around the lesions and to separate the mucosa from the lesions (E, H, G, I). During the process of peeling, hemostatic forceps were used to achieve hemostasis. The tumor was removed for pathological study and stained with narrow banding image (NBI) (D).

an improvement in endoscopic observations [7]. DMSO is a colorless hygroscopic liquid, and it has been used widely in several human diseases and it has been demonstrated to be effective and safe for the treatment of amyloidosis [8]. In our current report, oral DMSO was administered after ESD and the patient had no complaints during the follow-up period.

With advances in therapeutic endoscopy in recent years, the development of novel therapeutic strategies for gastric lesions using ESD has developed to enable the resection of different neoplasms with large sizes, irregular shapes, coexisting ulceration and difficult locations. En bloc resection is required in cases of PGLA [9]. The technique is advantageous in that it enables the direct dissection of the lesion.

Figure 4 The gastroscopic result 12 weeks after the endoscopic submucosal dissection (ESD). After ESD, lesions were observed at the gastric fundus, lesser curvature of the gastric antrum and angle of the stomach, and the surrounding mucosa was hyperemic. No active bleeding or fistulae were observed, and the surrounding mucosa of the gastric lesions was swollen, reddish and thickened.

Please cite this article in press as: Jin S-Z, et al. Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis. Clin Res Hepatol Gastroenterol (2014), http://dx.doi.org/10.1016/j.clinre.2014.01.002

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Letter to the editor In conclusion, the combination of ESD surgery and oral administration of DMSO was found to be feasible and safe as a novel strategy for the treatment for PGLA in a single patient.

Disclosure of interest The authors declare that they have no conflicts of interest concerning this article.

Acknowledgments We thank Medjaden Bioscience Limited for assisting in the preparation of this manuscript.

References [1] Ikeda S, Tojo K, Suzuki-Tsuchiya A, Akamatu T, Hashimoto T, Hiquchi K. Significant deposition of wild type transthyretinderived amyloid in the gastrointestinal tract of aged individuals. Amyloid 2009;16:174—7. [2] Kumar S, Dispenzieri A, Lacy MQ, Litzow MR, Gertz MA. High incidence of gastrointestinal tract bleeding after autologous stem cell transplant for primary systemic amyloidosis. Bone Marrow Transplant 2001;28:381—5. [3] Deniz K, Sari I, Torun E, Patiroqlu TE. Localized gastric amyloidosis: a case report. Turk J Gastroenterol 2006;17:116—9. [4] Sumiyama K, Tajiri H, Gostout CJ. Chemically assisted submucosal injection facilitates endoscopic submucosal dissection of gastric neoplasms. Endoscopy 2010;42:627—32. [5] Sugimoto T, Okamoto M, Mitsuno Y. Endoscopic submucosal dissection is an effective and safe therapy for early gastric

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[6]

[7]

[8] [9]

neoplasms: a multicenter feasible study. J Clin Gastroenterol 2012;46:124—9. Sattianayagam PT, Hawkins PN, Gillmore JD. Systemic amyloidosis and the gastrointestinal tract. Nat Rev Gastroenterol Hepatol 2009;6:608—17. Baird-Howell MA, Wurzel J. Fatal gastrointestinal hemorrhage in a paraplegic man with undiagnosed AA (secondary) amyloidosis. Amyloid 2011;18:245—8. Lee YN, Hong SJ, Kim HK. Localized gastric amyloidosis. Korean J Gastroenterol 2011;58:117—20. Uedo N, Iishi H, Tatsuta M. Long-term outcomes after endoscopic mucosal resection for early gastric cancer. Gastric Cancer 2006;9:88—92.

Shi-Zhu Jin a,1 Bo Qu a,1 Ming-Zi Han a Yan-Qiu Cheng a Guo-Ying Liang b Yan-Jie Chu a Fang Zhu a Bing-Rong Liu a,∗ a Department of Gastroenteroloy and Hepatology, Second Affiliated Hospital, Harbin Medical University, 150086 Harbin, China b Department of Gastroenteroloy and Hepatology, First Affiliated Hospital of Hei Long Jiang, University of Traditional Chinese Medicine, 150040 Harbin, China ∗

Corresponding author. Tel.: +86 0451 86605143. E-mail address: [email protected] (B.-R. Liu) 1 These authors contributed equally to the manuscript.

Please cite this article in press as: Jin S-Z, et al. Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis. Clin Res Hepatol Gastroenterol (2014), http://dx.doi.org/10.1016/j.clinre.2014.01.002

Endoscopic submucosal dissection combined with orally administered dimethyl sulfoxide for primary gastric localized amyloidosis.

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